Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hairy cell leukemia is a chronic but steadily progressive malignancy usually of older males. Clinically, patients present with splenomegaly and/or cytopenia. The diagnosis is made by demonstration of the hairy cell in Wright's-stained peripheral blood and in bone marrow and by the characteristic histologic findings in the bone marrow biopsy and spleen sections. Infection is the most significant problem complicating the course of patients with hairy cell leukemia and the role of granulocytopenia and/or monocytopenia is undoubtedly substantial. Splenectomy has produced an objective response in improving hematologic parameters in a large number of patients and may prolong survival in those patients who respond. The precise nature of hairy cells remains unknown. The cells exhibit features of both monocytes and B-lymphocytes in morphologic, cytochemical, immunologic and functional studies. A more complete understanding of the monocytic and lymphocytic stem cells and their maturation may provide insight into the origin of the hairy cell.
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PMID:Hairy cell leukemia: a review. 8 94

A 64-year-old man who had a two year history of hairy cell leukemia became feverish with night sweats. Splenomegaly and enlarged nodes in the neck and in the anterior mediastinum were detected. Splenectomy was performed and multiple white nodules on a dark-red background were seen in the spleen. The white nodules represented a large cell malignant lymphoma; other areas of the spleen contained mononuclear cells typical in hairy cell leukemia. One of the hilar lymph nodes of the spleen was involved by malignant lymphoma, and the other eight lymph nodes were involved by hairy cell leukemia. The coincidence of the two diseases tends to favor the view that hairy cells are of lymphocytic origin.
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PMID:Coincidental hairy cell leukemia and large cell malignant lymphoma. 31 72

A laboratory and clinical evaluation of 24 patients with hairy cell leukemia was carried out over a 23-month period. Most patients had splenomegaly without adenopathy or pancyotpenia. Nine of the patients had undergone splenectomy prior to referral; their median WBC count was 6600/mm3. The median WBC count for the 14 patients who had no prior therapy was 3550/mm3, and their median platelet count was 80,500/mm3. Spleen weights ranged from 618 to 3780 g; there appeared to be no relationship between the size of the spleen and the response in the blood counts after splenectomy. Four patients in whom the majority of the WBC were hairy cells underwent splenectomy, which produced no real change in their WBC count; however, there was improvement in the platelet count in three. In contrast, the presence of leukopenia with a low percentage of hairy cells predicted a beneficial response to splenectomy. The study of surface immunoglobulins (SIg) in 16 patients demonstrated that resynthesis had occurred in each case. Phagocytosis of zymosan was studied in 15 patients; in 8 of these, 25% or more of the hair cells were capable of phagocytosis; in 6 others, 0--9%; and in one, 13%. The resynthesis of SIg is a feature usually associated with B-lymphocytes, but the phagocytosis of zymosan is not. Thus, the existence of either a spectrum of functional capabilities of hairy cells or several distinct subtypes is suggested by these data. Platelet aggregation with epinephrine was abnormal in 7 of 14 patients studied but there were no clinically significant bleeding problems. A chromosome abnormality was present in 2 of the 19 patients from whom adequate samples were obtained; the abnormality probably involved chromsome 12 in both patients as well as absent Y and was associated with a rapidly progressive clinical course. The presence of a predominant number of hairy cells with a normal or increased peripheral blood WBC count or of a chromosomal abnormality suggests that splenectomy might not be beneficial as the initial therapy and that chemotherapy should be considered.
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PMID:Hairy cell leukemia: an unusual lymphoproliferative disease: a study of 24 patients. 35 59

A 65-year-old woman had pancytopenia, splenomegaly, and an inaspirable bone marrow. Diagnostic evaluation demonstrated that she had both leukemic reticuloendotheliosis (LRE), or hairy cell leukemia, and an additional lympho-reticular neoplasm, most likely a "histiocytic" lymphoma. The diagnosis of LRE was based on the histopathology of spleen tissue and of a bone marrow biopsy specimen. The diagnosis of diffuse "histiocytic" lymphoma was based on the histopathology of a splenic hilar and a mesenteric lymph node, tumor nodules in the kidney and spleen, and tissue from a mass obstructing a ureter. This is the first well-documented association of a second lympho-reticular neoplasm with LRE. Even relatively gently treatment of the "histiocytic" lymphoma resulted in fatal pancytopenia, illustrating the restricitons on therapy imposed by the marrow impairment due to the LRE.
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PMID:Coexistence of leukemic reticuloendotheliosis and histiocytic lymphoma: a case report. 37

Leukemic reticuloendotheliosis, or "hairy cell leukemia," is a neoplasm of the reticuloendothelial system. It is characterized by the presence of many "hairy cells" in blood, marrow, lymph nodes, and spleen; by anemia, leukopenia, thrombocytopenia, and often, by massive splenomegaly. Three such patients with spontaneous rupture and one patient with multiple infarctions of the spleen all had spleens which were large, congested, and diffusely infiltrated by "hairy cells." The lacerations in all three ruptured spleens were located in areas of extensive infarction. Indications for splenectomy in this disease include hypersplenism, severe cytopenia, hemodilution, splenomegaly with severe pressure symptoms, massive splenomegaly, rupture, or infarction.
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PMID:Spontaneous rupture of spleen in leukemic reticuloendotheliosis. 42 89

Enzymaticaly homogeneous fractions of lymphocytes, monocytes, and neutrophils were isolated by zonal centrifugation from peripheral blood of a patient with hairy cell leukemia, or leukemic reticuloendotheliosis, LRE,(with leukopenia, neutropenia, lymphocytosis, and massive splenomegaly). To detect enzymatic deficiencies, the cells were analyzed quantitatively for six leukocytic enzymes on three occasions: 1) before splenectomy, 2) 5 days after splenectomy, and 3) 6 weeks after splenectomy. Before splenectomy, the patient's cells showed moderate deficiency of beta-glucuronidase in lymphocytes and monocytes; server to modorate deficiency of lysozyme and myeloperoxidase in monocytes and granulocytes; and complete absence of neutral protease and alkaline phosphates in neutrophils. Full restoration of neutral protease and a three-fold rise in alkaline phosphatase activities occurred in the patient's neutrophils 5 days after splenectomy. Lysozyme and myeloperoxidase returned to normal in both monocytes and neutrophils of the patient. Six weeks following splenectomy, the alkaline phosphatase activity again disappeared from patient's neutrophils, although neutral protease remained normal. The patient's lymphocytes were unresponsive to PHA and PW mitogen before splenectomy but became responsive 6 weeks postoperatively. Monocytic transfomation into macrophges was supressed before and after splenectomy. The findings indicate that developmenally, in lymphocytic leukemia, a biochemical defect involves the patient's monocytes and neutrophils much more severely than it affects the leukemic lymphocytes. Functionally, the results partly explain the susceptibility of LRE patients to microbial infections.
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PMID:Absence of neutral protease and alkaline phosphatase in neutrophils of a case of hairy cell leukemia. 43 13

The experience of the University of Colorado Medical Center affiliated hospitals with leukemic reticuloendotheliosis, hairy cell leukemia, during the past two years has been reviewed. Eight instances were found. The majority of patients in this study presented with fatigue, pancytopenia and splenomegaly. Diagnosis was based upon finding characteristic hairy cells in the blood, bone marrow or spleen. Treatment, unlike other hematopoietic malignant conditions, was primarily surgical, with splenectomy being the treatment of choice, which may lead to prolonged remission in the majority of instances.
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PMID:Surgical leukemia. 68 93

We studied the clinical and laboratory characteristics of 71 patients with hairy cell leukemia to ascertain whether prognostic factors important for the therapy of the disease existed. Granulocytopenia of less than 500/mu 1 was associated with the occurrence of a serious infection in 46% of the patients, compared to 19% of patients whose absolute granulocyte count was greater than 500/mu 1 (P less than 0.05). In most patients with symptoms of thrombocytopenia or leukopenia, splenectomy led to improved blood counts. Patients treated with splenectomy only had a significantly longer survival than those treated with steroids only (P less than 0.05). A group of patients who were slightly older and had minimal splenomegaly and few hairy cells in the peripheral blood did well without therapeutic intervention. Twenty-four patients have died, 16 of an infectious process. The actuarial survival for the group is 51% at 4 years, and there has been no change subsequently.
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PMID:Hairy cell leukemia: a clinical review based on 71 cases. 71 40

Chronical course, splenomegaly and pancytopenia are characteristic for hairy cell leukemia. The main feature are so-called hairy cells in peripheral blood. This cells were investigated by cytological, cytochemical, histological and electron microscopical methods. Special cytoplasmic inculsions seem to be specific for this cell type.
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PMID:[Hairy cell leukemia]. 80 69

2 cases of hairy cell leukemia are reported. The disease is important for pathologists, because the splenomegaly is the leading clinical sign and often a splenectomy is performed with infomation about the true disease. The morphology of the spleen with infiltration of pulp cords and filling of sinuses by monomorphous blasts, with moderate fibrosis and atrophy of the white pulp is not convincing, because the hairy-like processes of the toumor-cell cytoplasm are not seen by usual histological examination. Hemocytomorphology and -cytochemistry are essential diagnostic procedures.
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PMID:[Hairy cell leukemia (author's transl)]. 84 70


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